Leucémie à tricholeucocytes

Abstract

Summary Hairy cell leukemia (HCL) accounts for 2% of all haematologic cancers. The diagnosis is based on the identification of hairy cells in the peripheral blood, bone marrow and/orspleen. Hairy cells are B-cells, which express CD11c, CD25, CD103, DBA-44 and CD123 without expressing CD5. No specific genetic abnormality was described in HCL and it is necessary to distinguish HCL from all other B-cell chronic lymphoproliferative disorders, specially splenic lymphoma with villous lymphocytes (SLVL) and the variant form of HCL (HCL-V) because specific treatments are required. All the immunologic and molecular data suggest that hairy cell is a postgerminal center memory B-cell. Purine analogs, 2’-deoxycoformycin (DCF) or 2-chloro-deoxyadenosine are very active and used as first line treatment. DCF changed the natural history of HCL: however the risk of cytopenia, immunosuppression and second cancer must be evaluated carefully. Eradication of minimal residual disease (MRD) in HCL using monoclonal antibodies (rituximab) after therapy with nucleoside analogs is feasible.